{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,30]],"date-time":"2026-09-30T13:31:26Z","timestamp":1790775086931,"version":"4.1.0"},"reference-count":21,"publisher":"Ovid Technologies (Wolters Kluwer Health)","issue":"1","content-domain":{"domain":["www.ahajournals.org"],"crossmark-restriction":true},"short-container-title":["Circulation"],"published-print":{"date-parts":[[2018,7,3]]},"abstract":"<jats:sec>\n                    <jats:title>Background:<\/jats:title>\n                    <jats:p>Late survival and symptomatic status of children with hypertrophic cardiomyopathy have not been well defined. We examined long-term outcomes for pediatric hypertrophic cardiomyopathy.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods:<\/jats:title>\n                    <jats:p>The National Australian Childhood Cardiomyopathy Study is a longitudinal population-based cohort study of children (0\u201310 years of age) diagnosed with cardiomyopathy between 1987 and 1996. The primary study end point was time to death or cardiac transplantation.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results:<\/jats:title>\n                    <jats:p>\n                      There were 80 patients with hypertrophic cardiomyopathy, with a median age at diagnosis of 0.48 (interquartile range, 0.1, 2.5) years. Freedom from death\/transplantation was 86% (95% confidence interval [CI], 77.0\u201392.0) 1 year after presentation, 80% (95% CI, 69.0\u201387.0) at 10 years, and 78% (95% CI, 67.0\u201386.0) at 20 years. From multivariable analyses, risk factors for death\/transplantation included symmetrical left ventricular hypertrophy at the time of diagnosis (hazard ratio, 4.20; 95% CI, 1.60\u201311.05;\n                      <jats:italic>P<\/jats:italic>\n                      =0.004), Noonan syndrome (hazard ratio, 2.88; 95% CI, 1.02\u20138.08;\n                      <jats:italic>P<\/jats:italic>\n                      =0.045), higher posterior wall thickness\n                      <jats:italic>z<\/jats:italic>\n                      score (hazard ratio, 1.45; 95% CI, 1.22\u20131.73;\n                      <jats:italic>P<\/jats:italic>\n                      &lt;0.001), and lower fractional shortening\n                      <jats:italic>z<\/jats:italic>\n                      score (hazard ratio, 0.84; 95% CI, 0.74\u20130.95;\n                      <jats:italic>P<\/jats:italic>\n                      =0.005) during follow-up. Nineteen (23%) subjects underwent left ventricular myectomy. At a median of 15.7 years of follow-up, 27 (42%) of 63 survivors were treated with \u03b2-blocker, and 13 (21%) had an implantable cardioverter-defibrillator.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions:<\/jats:title>\n                    <jats:p>The highest risk of death or transplantation for children with hypertrophic cardiomyopathy is within 1 year after diagnosis, with low attrition rates thereafter. Many subjects receive medical, surgical, or device therapy.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1161\/circulationaha.117.028895","type":"journal-article","created":{"date-parts":[[2018,2,28]],"date-time":"2018-02-28T05:25:15Z","timestamp":1519795515000},"page":"29-36","update-policy":"https:\/\/doi.org\/10.1161\/crossmarkpolicy","source":"Crossref","is-referenced-by-count":105,"title":["Long-Term Outcomes of Hypertrophic Cardiomyopathy Diagnosed During Childhood"],"prefix":"10.1161","volume":"138","author":[{"given":"Peta M.A.","family":"Alexander","sequence":"first","affiliation":[{"name":"Royal Children\u2019s Hospital, Melbourne, Australia (P.M.A.A., A.M.D., R.G.W.)"},{"name":"Murdoch Children\u2019s Research Institute, Melbourne, Australia (P.M.A.A., K.J.L., T.S., A.M.D., I.K., R.G.W.)"},{"name":"Boston Children\u2019s Hospital, MA (P.M.A.A., L.A.S., S.D.C.)"},{"name":"Harvard Medical School, Boston, MA (P.M.A.A., L.A.S., S.D.C.)"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alan W.","family":"Nugent","sequence":"additional","affiliation":[{"name":"University of Texas Southwestern Medical Center, Dallas (A.W.N.)"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Piers E.F.","family":"Daubeney","sequence":"additional","affiliation":[{"name":"Royal Brompton Hospital, London, United Kingdom (P.E.F.D.)"},{"name":"National Heart and Lung Institute, Imperial College London, United Kingdom (P.E.F.D.)"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Katherine J.","family":"Lee","sequence":"additional","affiliation":[{"name":"Murdoch Children\u2019s Research Institute, Melbourne, Australia (P.M.A.A., K.J.L., T.S., A.M.D., I.K., R.G.W.)"},{"name":"University of Melbourne, Australia (K.J.L., A.M.D., R.G.W.)"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lynn A.","family":"Sleeper","sequence":"additional","affiliation":[{"name":"Boston Children\u2019s Hospital, MA (P.M.A.A., L.A.S., S.D.C.)"},{"name":"Harvard Medical School, Boston, MA (P.M.A.A., L.A.S., S.D.C.)"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tibor","family":"Schuster","sequence":"additional","affiliation":[{"name":"Murdoch Children\u2019s Research Institute, Melbourne, Australia (P.M.A.A., K.J.L., T.S., A.M.D., I.K., R.G.W.)"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Christian","family":"Turner","sequence":"additional","affiliation":[{"name":"Children\u2019s Hospital at Westmead, Sydney, Australia (C.T., G.F.S.)"},{"name":"University of Sydney, Australia (C.T., G.F.S.)"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Andrew M.","family":"Davis","sequence":"additional","affiliation":[{"name":"Royal Children\u2019s Hospital, Melbourne, Australia (P.M.A.A., A.M.D., R.G.W.)"},{"name":"Murdoch Children\u2019s Research Institute, Melbourne, Australia (P.M.A.A., K.J.L., T.S., A.M.D., I.K., R.G.W.)"},{"name":"University of Melbourne, Australia (K.J.L., A.M.D., R.G.W.)"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Chris","family":"Semsarian","sequence":"additional","affiliation":[{"name":"Agnes Ginges Centre for Molecular Cardiology, Centenary Institute, University of Sydney, Australia (C.S.)"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Steven D.","family":"Colan","sequence":"additional","affiliation":[{"name":"Boston Children\u2019s Hospital, MA (P.M.A.A., L.A.S., S.D.C.)"},{"name":"Harvard Medical School, Boston, MA (P.M.A.A., L.A.S., S.D.C.)"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Terry","family":"Robertson","sequence":"additional","affiliation":[{"name":"Women\u2019s and Children\u2019s Hospital, Adelaide, Australia (T.R.)"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"James","family":"Ramsay","sequence":"additional","affiliation":[{"name":"Princess Margaret Hospital, Perth, Australia (J.R.)"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Robert","family":"Justo","sequence":"additional","affiliation":[{"name":"University of Queensland, Brisbane, Australia (R.J.)."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gary F.","family":"Sholler","sequence":"additional","affiliation":[{"name":"Children\u2019s Hospital at Westmead, Sydney, Australia (C.T., G.F.S.)"},{"name":"University of Sydney, Australia (C.T., G.F.S.)"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ingrid","family":"King","sequence":"additional","affiliation":[{"name":"Murdoch Children\u2019s Research Institute, Melbourne, Australia (P.M.A.A., K.J.L., T.S., A.M.D., I.K., R.G.W.)"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Robert G.","family":"Weintraub","sequence":"additional","affiliation":[{"name":"Royal Children\u2019s Hospital, Melbourne, Australia (P.M.A.A., A.M.D., R.G.W.)"},{"name":"Murdoch Children\u2019s Research Institute, 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